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論文
- タイトル
- タイトル（英）
- The N-Terminal Fragment of Urine Titin Is Not a Product of Degradation by Calpain 3.
- 参照URL
- https://researchmap.jp/read0208506/published_papers/49802585
- 著者
- 著者（英）
- Yoshinori Nambu,Matsumura T,Machida K,Tsutsumi R,Hata S,Shinkai-Ouchi F,Ono Y,Osawa K,Shirakawa T,Bo R,Nishio H,Sakaue H,Hiroyuki Awano,Matsuo M
- 担当区分
- 概要
- 概要（英）
- <h4>Introduction</h4>A 20 kDa fragment at the N-terminus of titin is highly excreted in the urine of patients with Duchenne muscular dystrophy (DMD), making urine titin a prominent biomarker for muscle breakdown. This N-terminal fragment is presumed to be a product of degradation by a protein-degrading enzyme, calpain 3; however, whether calpain 3 is required remains unclear. We aimed to determine whether urine titin elevation occurs in the absence of calpain 3.<h4>Methods</h4>We measured urine titin by ELISA in two genetically confirmed limb-girdle muscular dystrophy type R1(LGMDR1) patients, 11 other LGMD patients, and five healthy controls. Five Capn3-/- and nine wild-type mice were also examined.<h4>Results</h4>Urine titin in LGMDR1 patients was ~100-fold higher than in controls (median 112.3 vs. 1.3 pmol/mg Cr, p < 0.0001), with no difference between LGMDR1 and other LGMD subtypes. Similarly, urine titin levels in Capn3-/- mice were more than four times higher than normal (p < 0.01).<h4>Discussion</h4>These results suggest the involvement of other protein-degrading enzymes leading to the production of the N-terminal fragment.
- 出版者・発行元
- 出版者・発行元（英）
- 誌名
- 誌名（英）
- Muscle & nerve
- 巻
- 号
- 開始ページ
- 終了ページ
- 出版年月
- 2025年1月8日
- 査読の有無
- 査読有り
- 招待の有無
- 掲載種別
- 研究論文（学術雑誌）
- ISSN
- DOI URL
- https://doi.org/10.1002/mus.28340
- 共同研究・競争的資金等の研究課題